Moyamoya Disease: Advances in Diagnosis, Treatment, and Surgical Interventions

Abstract

Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by progressive narrowing of the brain's arteries, leading to an increased risk of stroke. The primary susceptibility gene, RNF213, has been identified in individuals of East Asian descent, contributing to ongoing research into potential therapeutic targets. The distinction between idiopathic MMD and secondary forms, such as Moyamoya syndrome (MMS), is discussed, focusing on associated conditions and risk factors. Surgical revascularization emerges as the mainstay of treatment, with direct, indirect, and combination bypass procedures explored. The review delves into advancements in imaging technology for diagnosis and treatment planning, emphasizing non-invasive magnetic resonance examination's role in identifying asymptomatic patients. Additionally, insights into anesthetic care and therapeutic approaches underscore the evolving understanding of this complex disease. The presented information aims to contribute to the ongoing dialogue surrounding MMD, providing a valuable resource for clinicians and researchers.

Keywords: cerebral revascularization; cerebrovascular disorders; intracranial arterial diseases; moyamoya disease; neurosurgical procedures.

Figure 1. PRISMA flow diagram

PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses